Journal
JOURNAL OF INFLAMMATION RESEARCH
Volume 15, Issue -, Pages 5739-5755Publisher
DOVE MEDICAL PRESS LTD
DOI: 10.2147/JIR.S343261
Keywords
autoinflammation; adult onset Still?s disease; inflammasome; VEXAS; Schnitzler?s syndrome
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This review discusses new developments in acquired/late onset SAID, including various diseases such as phenocopies, Schnitzler's syndrome, Adult onset Still's disease, VEXAS syndrome, and autoinflammatory complications associated with myelo-dysplastic syndrome.
Systemic autoinflammatory diseases (SAID) are conditions caused by dysregulation or disturbance of the innate immune system, with neutrophils and macrophages the main effector cells. Although there are now more than 40 distinct, genetically defined SAIDs, the genetic/molecular diagnosis remains unknown for a significant proportion of patients with the disease onset in adulthood. This review focuses on new developments related to acquired/late onset SAID, including phenocopies of monogenic disorders, Schnitzler's syndrome, Adult onset Still's disease, VEXAS syndrome, and autoinflammatory complications associated with myelo-dysplastic syndrome.
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